A new global playbook for a rare and complex cancer: updated global consensus on chordoma

In July 2026, JAMA Oncology published an updated Global Consensus on the Management of Primary Localized Chordoma—the product of a June 2025 meeting in Milan that brought together more than 150 specialists spanning surgery, radiation oncology, pathology, medical oncology, rehabilitation medicine, and palliative care, alongside patients and patient advocates, organized by the Chordoma Foundation. 

The result is a more detailed, site-specific framework than its predecessor.

Why an update was needed

The 2015 consensus offered broad, principle-based guidance covering both localized and metastatic disease. In the decade since, imaging protocols have improved, molecular markers such as brachyury and SMARCB1 have been incorporated into subtype characterization, and surgical techniques and radiotherapy—particularly particle therapy—have advanced. The new statement reflects these developments while narrowing its focus specifically to primary, localized disease and organizing its recommendations by anatomical site: skull base, mobile spine, and sacrum.

Site-specific guidance, not one-size-fits-all

Perhaps the most significant shift from 2015 is the move away from generalized treatment principles toward structured, decision-tree-style pathways for each anatomical location.

Skull base chordoma. The consensus introduces the emerging concept of supramarginal resection—including dural and bony margins when involved—when this can be performed without significantly increasing surgical morbidity. It also provides detailed guidance on endoscopic and transcranial surgical approaches, with approach selection based on tumor location and extent, and specifies high-dose radiotherapy guidance, including doses of 74 Gy equivalents or more in relevant treatment volumes.

Mobile spine chordoma. Here, the guidance centers on en bloc resection with negative margins as the goal, careful biopsy technique to avoid tumor seeding along the needle tract, and a recommendation to favor carbon fiber or titanium hardware for spinal reconstruction when possible—materials selected because they minimize postoperative imaging artifacts and avoid interference with radiation planning and delivery.

Sacral chordoma. The sacrum guidance weighs oncologic control against the very real risk of bowel, bladder, sexual, and lower-limb dysfunction depending on the extent of sacral and nerve involvement. Notably, based on favorable outcomes from Japanese centers and interim results from the SACRO trial showing noninferiority to surgery, the consensus states that definitive proton or carbon-ion radiotherapy may be offered as a primary treatment for selected patients—not only as an option for unresectable disease. Selection should take into account treatment-related toxicity as well as patient-specific and tumor-specific factors.

A more nuanced role for systemic therapy

One of the clearer updates concerns chemotherapy. For conventional chordoma—the most common subtype—the consensus is clear: chemotherapy is not recommended in the neoadjuvant or adjuvant setting for localized disease amenable to curative surgical resection and/or radiotherapy, given its negligible demonstrated efficacy.

For the rarer and more aggressive poorly differentiated and dedifferentiated subtypes, systemic therapy has a more nuanced role. Poorly differentiated chordoma may have greater chemosensitivity, and when it is confirmed, chemotherapy should be considered, preferably preoperatively, to assess response and facilitate local control. For dedifferentiated chordoma, evidence remains limited, but its behavior may mirror that of high-grade sarcoma, so chemotherapy can be considered. Because of uncertain benefit and potential toxic effects, systemic therapy should be considered selectively in patients with good performance status.

Life after treatment: follow-up and quality of life

The new consensus also formalizes risk-adapted, long-term surveillance. Conventional chordoma patients face imaging surveillance for at least 15 years given how late recurrences can occur, with the consensus citing a median of 4 years for local recurrence and 3 years for distant recurrence. MRI of the primary site is recommended every 6 to 12 months for the first 4 to 5 years, followed by every 1 to 2 years for at least 15 years.

Poorly differentiated and dedifferentiated subtypes, with their higher metastatic potential, are followed on a more intensive schedule modeled on high-grade bone sarcoma protocols, including more frequent imaging of the primary site and assessment for distant disease.

Just as importantly, the consensus dedicates structured attention to palliative and rehabilitative care—addressing chronic pain, neurological and musculoskeletal impairment, and site-specific functional loss—reflecting the importance of quality of life alongside disease control for chordoma patients, many of whom live with the disease and its treatment consequences for years or decades.

The bigger picture

What stands out most about this update isn't any single recommendation—it's the process behind it. Chordoma's rarity means no single center, or even country, sees enough cases to generate the evidence base that more common cancers enjoy. This consensus represents what is possible when specialists across disciplines and countries—together with patients and patient advocates—pool their collective experience and the available evidence into a single, actionable framework.

The authors are candid that important gaps remain. Recurrent disease remains challenging and, as the authors note, will require a dedicated future consensus. Priorities for future research include prognostic biomarkers, advances in particle therapy, reirradiation, function-preserving surgery, and targeted therapies, with patient-reported outcomes also identified as an important component of future care and trials.

For now, clinicians managing primary localized chordoma have a substantially more detailed framework than they did a year ago—one grounded in the available evidence, multidisciplinary consensus, and the accumulated experience of specialists caring for this rare disease.